Advertisement

The Ross/Ross-Konno procedure in infancy is a safe and durable solution for aortic stenosis

Published:September 07, 2021DOI:https://doi.org/10.1016/j.jtcvs.2021.06.066

      Abstract

      Objective

      The objective of this study was to characterize early and midterm outcomes after the Ross/Ross-Konno procedure performed in infancy for severe aortic valve disease.

      Methods

      Between January 1995 and December 2018, 35 infants younger than 1 year (13 neonates) underwent a Ross/Ross-Konno procedure. Patients were followed up to a median of 4.1 years (interquartile range [IQR], 2.6-9.5). Primary outcome measures were survival, early morbidity, freedom from reintervention and long-term functional and echocardiographic status.

      Results

      Median age at operation was 49 days (IQR, 17-135) and weight was 4 kg (IQR, 3.4-5.2). Thirty-one (89%) had undergone a previous procedure, including balloon valvuloplasty in 26 (74%). Thirty (86%) required annular enlargement (Konno incision). Five required concomitant aortic arch surgery (2 neonates, 3 infants). There were no early deaths, and 1 late death at 18 months. Freedom from reoperation was 85% (95% confidence interval [CI], 68%-93%) at 1 year, 76% (95% CI, 54%-88%) at 5 years, and 62% (95% CI, 36%-79%) at 10 years. One modified Konno was performed at 5 years after a Ross in infancy. Ten right ventricle to pulmonary artery conduits have required reintervention (2 percutaneous pulmonary valve implantations). One child required a permanent pacemaker for complete heart block. At latest follow-up, 32 (94%) of 34 survivors were asymptomatic. There was no significant change in neoaortic Z-scores between 6 weeks and latest follow-up.

      Conclusions

      The neonatal and infant Ross/Ross-Konno procedure can be performed with low mortality and achieves a stable left ventricular outflow tract. Significant early morbidity reflects the preoperative condition of the patients but definitive surgery of this type can be considered as a primary approach.

      Graphical abstract

      Key Words

      Abbreviations and Acronyms:

      CI (confidence interval), ECMO (extracorporeal membrane oxygenation), IQR (interquartile range), MCS (mechanical circulatory support), RV (right ventricle), RV-PA (right ventricle to pulmonary artery), SD (standard deviation)
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to The Journal of Thoracic and Cardiovascular Surgery
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Ross D.N.
        Replacement of aortic and mitral valves with a pulmonary homograft.
        Lancet. 1967; 290: 956-958
        • Elkins R.C.
        • Knott-Craig C.J.
        • Ward K.E.
        • McCue C.
        • Lane M.M.
        Pulmonary autograft in children: realized growth potential.
        Ann Thorac Surg. 1994; 57: 1387-1393
        • Horer J.
        • Hanke T.
        • Stierle U.
        • Takkenberg J.J.
        • Bogers A.J.
        • Hemmer W.
        • et al.
        Neoaortic root diameters and aortic regurgitation in children after the Ross operation.
        Ann Thorac Surg. 2009; 88: 594-600
        • Tantengco M.V.
        • Humes R.A.
        • Clapp S.K.
        • Lobdell K.W.
        • Walters H.L.
        • Hakimi M.
        • et al.
        Aortic root dilation after the Ross procedure.
        Am J Cardiol. 1999; 83: 915-920
        • Solowiejczyk D.E.
        • Bourlon F.
        • Apfel H.D.
        • Hordof A.J.
        • Hsu D.T.
        • Crabtree G.
        • et al.
        Serial echocardiographic measurements of the pulmonary autograft in the aortic valve position after the Ross operation in a pediatric population using normal pulmonary artery dimensions as the reference standard.
        Am J Cardiol. 2000; 85: 1119-1123
        • Pradegan N.
        • Castaldi B.
        • Azzolina D.
        • Stellin G.
        • Vida V.L.
        Long-term fate of the neoaortic root after neonatal Ross operation: a case series.
        World J Pediatr Congenit Heart Surg. 2019; 10: 364-366
        • Shinkawa T.
        • Bove E.L.
        • Hirsch J.C.
        • Devaney E.J.
        • Ohye R.G.
        Intermediate-term results of the Ross procedure in neonates and infants.
        Ann Thorac Surg. 2010; 89: 1827-1832
        • Mookhoek A.
        • Charitos E.I.
        • Hazekamp M.G.
        • Bogers A.J.
        • Horer J.
        • Lange R.
        • et al.
        Ross procedure in neonates and infants: a European multicenter experience.
        Ann Thorac Surg. 2015; 100: 2278-2284
        • Elder R.W.
        • Quaegebeur J.M.
        • Bacha E.A.
        • Chen J.M.
        • Bourlon F.
        • Williams I.A.
        Outcomes of the infant Ross procedure for congenital aortic stenosis followed into adolescence.
        J Thorac Cardiovasc Surg. 2013; 145: 1504-1511
        • Luciani G.B.
        • Lucchese G.
        • Carotti A.
        • Brancaccio G.
        • Abbruzzese P.
        • Caianiello G.
        • et al.
        Two decades of experience with the Ross operation in neonates, infants and children from the Italian Paediatric Ross registry.
        Heart. 2014; 100: 1954-1959
        • Sames-Dolzer E.
        • Wickenhauser E.
        • Kreuzer M.
        • Benedikt P.
        • Gitter R.
        • Prandstetter C.
        • et al.
        The Ross-Konno procedure in neonates and infants less than 3 months of age.
        Eur J Cardiothorac Surg. 2018; 54: 71-77
        • Nelson J.S.
        • Pasquali S.K.
        • Pratt C.N.
        • Yu S.
        • Donohue J.E.
        • Loccoh E.
        • et al.
        Long-term survival and reintervention after the Ross procedure across the pediatric age spectrum.
        Ann Thorac Surg. 2015; 99: 2086-2094
        • Lo Rito M.
        • Davies B.
        • Brawn W.J.
        • Jones T.J.
        • Khan N.
        • Stickley J.
        • et al.
        Comparison of the Ross/Ross-Konno aortic root in children before and after the age of 18 months.
        Eur J Cardiothorac Surg. 2014; 46: 450-457
        • Donald J.S.
        • Wallace F.R.O.
        • Naimo P.S.
        • Fricke T.A.
        • Brink J.
        • Brizard C.P.
        • et al.
        Ross operation in children: 23-year experience from a single institution.
        Ann Thorac Surg. 2020; 109: 1251-1259
        • Pettersen M.D.
        • Du W.
        • Skeens M.E.
        • Humes R.A.
        Regression equations for calculation of Z scores of cardiac structures in a large cohort of healthy infants, children, and adolescents: an echocardiographic study.
        J Am Soc Echocardiogr. 2008; 21: 922-934
        • Maeda K.
        • Rizal R.E.
        • Lavrsen M.
        • Malhotra S.P.
        • Akram S.A.
        • Davies R.
        • et al.
        Midterm results of the modified Ross/Konno procedure in neonates and infants.
        Ann Thorac Surg. 2012; 94: 156-162
        • Saxena A.
        • Salve G.G.
        • Betts K.
        • Arora N.
        • Cole A.D.
        • Sholler G.F.
        • et al.
        Outcomes following heterotopic placement of right ventricle to pulmonary artery conduits.
        World J Pediatr Congenit Heart Surg. 2021; 12: 220-229
        • Pasquali S.K.
        • Cohen M.S.
        • Shera D.
        • Wernovsky G.
        • Spray T.L.
        • Marino B.S.
        The relationship between neo-aortic root dilation, insufficiency, and reintervention following the Ross procedure in infants, children, and young adults.
        J Am Coll Cardiol. 2007; 49: 1806-1812

      Linked Article

      • Commentary: Ross procedure in neonates and infants: Withstanding the litmus test of time
        The Journal of Thoracic and Cardiovascular SurgeryVol. 163Issue 2
        • Preview
          More than 50 years after Donald Nixon Ross developed his eponymous procedure of replacing a diseased aortic valve with pulmonary autograft, the technique has become an established surgical option for aortic valve replacement.1 The Ross procedure offers superior hemodynamic parameters in comparison to alternative means for aortic valve replacement, avoidance of anticoagulation therapy, and growth potential for patients at younger ages. In the pediatric population, it is the sole option if valve repair is not feasible or fails.
        • Full-Text
        • PDF
      • Commentary: Ross procedure is indeed a safe and durable option for critical aortic valve disease in neonates and infants
        The Journal of Thoracic and Cardiovascular SurgeryVol. 163Issue 2
        • Preview
          Management of neonates and infants with critical aortic valve stenosis (AS) poses a significant challenge. The patients present with various degree of left ventricular dysfunction and endocardial fibroelastosis due to critical downstream obstruction, secondary pulmonary hypertension, and important additional lesions such as mitral valve dysplasia and aortic arch obstruction. Balloon aortic valvuloplasty has been a standard first intervention for these patients, but surgical intervention is needed when balloon valvuloplasty is ineffective or results in significant aortic insufficiency (AI).
        • Full-Text
        • PDF